| ObjectiveObservation of pyridostigmine and or gluconcorticoid therapy clinical efficacy and side effect in patients with MG children, and the changes of immune globulin, complement components and lymphocyte subsets in peripheral blood before and after treatment, to investigate immunological pathogenesis in MG children and drug effect on immune function.Method33 patients were selectde for this follow-up survey, who were newly diagnosed with MG at onset from January 2013 to September 2014 in Children’s Hospital of Chongqing Medical University.33 patients were used humoral immune function testing nephelometry:serum IgG, IgA, IgM and complement C3 and C4; 24 patients were used flow cytometry immune function cells:CD3+T cells, CD19+B cells, CD4+Th cells, CD8+Ts cells, CD56+CD16+NK cells, CD4+/CD8+ratio; all the indicators above were tested before treatment and after treatment at six months. Nine out of them were treated with oral pyridostigmine alone, while 24 patients were treated with oral pyridostigmine and gluconcorticoid.We follow up them at least 6 months. The follow up contents are improvement of clinical symptoms and adverse reactions after therapy in MG children.Result1. Among the 33 cases,16 were males and 17 females, giving the ratio of M/F around 1:1.1. The average age was 4.6±3.7 years old.2. Comparison of immune function between OMG and GMG:IgA was significantly higher in GMG children(t=-2.83, P<0.05), there were no significant differences between OMG and GMG at IgG, IgM and complement C3, C4 (t=-1.33-0.45, P> 0.05). There were no significant difference between OMG and GMG at the percentage of CD3+T cells, CD19+B cells, CD4+Th cells, CD8+Ts cells and CD56+CD16+NK cells, as well as the ratio of CD4+/CD8+in MG children (t=-1.10-1.40, P> 0.05).3. The changes of immune function before and after therapy in MG children:IgG, IgA, IgM were slightly elevated, C3, C4 were slightly lowered after therapy, but the differences were not statistically significant (t =-1.23-1.00, P> 0.05). The percentage of CD4+Th cells, CD 19+B cells and the ratio of CD4+/CD8+were decreased, the percentage of CD3+T cells, CD8+Ts cells and CD56+CD16+NK cells were increased, but the differences were also not statistically significant (t=-1.75-1.95, P> 0.05).4. Correlation between the distribution of immune function and progn-osis of MG after medication:IgG, IgA, IgM and complement C3, C4 in effective group were slightly higher than ones in ineffective group, but the differences were not statistically significant (t=-1.00～-0.27, P> 0.05). The percentage of CD 19+B cells was lower and the ratio of CD4+/CD8+ was higher in effective group than ones in ineffective group, the differences were statistically significant (t=-2.38～3.50, P<0.05).The percentage of CD3+T cells, CD4+Th cells and CD56+CD16+NK cell were slightly higher than ones in ineffective group, the percentage of CD8+Ts cell was slightly lower than that in ineffective group, but the differences were not statistically significant (t=-1.52-1.86, P> 0.05).5. Comparison of the efficiency rate in hormone group and none hormone group:the differences between gender and clinical type in two groups were not statistically significant (χ2= 1.24～1.64, P>0.05), and the differences between age in two groups were not statistically significant (t= 0.71, P>0.05), so they were homogeneous. The efficiency rate of hormone group was 91.7%, while the efficiency rate of none hormone group was 55.6%, the differences were statistically significant2= 5.74, P<0.05), suggesting that gluconcorticoid was more efficient.6. Comparison of the side effect rate in hormone group and none hormone group:24 cases in hormone group,18 patients (75.0%) appeared Cushing’s appearance, weight gain and other hormone-related adverse reactions, but the adverse reactions would gradually reduce, or even disappear because of the improvement of clinical symptoms in patients with steroid reduction; and no symptoms of MG were more serious than before, serious adverse reactions like myasthenic crisis, high blood pressure, severe infections and fractures would not occur.9 cases in pyridostigmine group, two cases (22.2%) occured with sweating, the differences were statistically significant (χ2= 7.64, P<0.05).Conclusion1. IgA was significantly higher in GMG children, suggesting that immunoglobulin may play a role on the incidence of MG in children.2. The percentage of CD 19+B cells was lower and the ratio of CD4+ /CD8+was higher in effective group than ones in ineffective group, the differences of IgG. IgA、IgM、C3、C、CD3+Tcells、CD4+Thcell、 CD8+Ts cells、CD56+CD16+NK cells between effective group and ineffective group were not significant,suggesting that MG children have immune function disorder in different degree,also the abnormal condition was various.Gluconcorticoid play a therapeutic role by improving and regulating the disorder of cellular immune function,so so as to alleviate the symptoms of MG and prevent the relapse of MG.3. Gluconcorticoid was more efficient than pyridostigmine in MG children, although a transient Cushing’s appearance, weight gain and other adverse reactions, but the adverse reactions would gradually reduce, or even disappear because of the improvement of clinical symptoms in patients with steroid reduction. |
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